The forming of gallstones is a common problem for people with pyruvate kinase deficiency and may result in biliary colic.
What’s the link between gallstones and Pyruvate Kinase Deficiency?
Gallstones (also called cholelithiasis) are formed when there is a high level of a particular substance in the bile fluid. In patients with hemolytic anemia, there is a high concentration of bilirubin and calcium in the bile fluid and the concentration of the bile acids decreases. The high concentrations of bilirubin and calcium lead to an oversaturation of calciumbilirubinate in the bile. This calciumbilirubinaat precipitates and forms microscopic gallstones. As the saturation persists, gallstones grow into mature black pigment stones. These black pigment gallstones are very typical for patients with hemolytic anemia. They are not the same kind of gallstones as the brown pigment stones and cholesterol stones.
“When I was 16 my upperright tummy started to hurt a lot. Especially after eating peanuts. When they removed my gallblader it was very inflamed and the gallstone was the size and color of a giant meatball”
Female, 40 years
Gallbladder removal
To avoid gallstones, many patients with PKD have their gallbladder removed through laparoscopic surgery, which is called “cholecystectomy”. Mostly this happens in combination with the removal of the spleen.
“I had my gallbladder removed with laparoscopic surgery when I was 25. Up until then I had several smaller and bigger biliary colics a year and the pain used to hit me just below the chest”
Female, 28 years
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Pyruvate Kinase Deficiency 